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• W2093441092 abstract "<i>Background/Aims:</i> β^S-Haplotype prevalence and its associations with clinical and hematological characteristics were assessed in Brazilian children with sickle cell anemia or Sβ&#8304;-thalassemia. <i>Methods:</i> A retrospective randomized cohort study was undertaken with 208 SS and 13 Sβ&#8304;-thalassemia children derived from the Newborn Screening Program of the state of Minas Gerais. β^S-Haplotypes were determined by PCR-RFLP. <i>Results:</i> Thirty-nine percent of the SS subjects had the CAR/CAR genotype, 33% had CAR/Ben, 24% had Ben/Ben, 1% had CAR/Atp, 1% had Ben/Atp, and 1% had Arab-Indian/Ben; 1% could not be characterized. Of the Sβ&#8304;-thalassemia children, 5 were CAR/undefined, 2 were Ben/undefined, and 1 was CAM/undefined. There was no significant association between β^S-haplotypes and the total Hb, Hb F, MCV, MCH, WBC, and reticulocyte count among the SS children. Likewise, no significant association was detected between β^S-haplotypes and the frequency of acute chest syndrome episodes, blood transfusions, splenic sequestration, or cerebrovascular disease (high-risk/conditional transcranial Doppler ultrasonography or clinical stroke). A limited number of Sβ&#8304;-thalassemia children precluded valid analyses. <i>Conclusions:</i> The prevalence of β^S-haplotypes in this study is in agreement with the historical records of African slaves brought to the state of Minas Gerais. Furthermore, β^S-haplotypes CAR and Ben were not associated with any analyzed feature of children with sickle cell anemia." @default.
• W2093441092 created "2016-06-24" @default.
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• W2093441092 date "2010-01-01" @default.
• W2093441092 modified "2023-09-29" @default.
• W2093441092 title "β-Globin Gene Cluster Haplotypes in a Cohort of 221 Children with Sickle Cell Anemia or Sβ&#8304;-Thalassemia and Their Association with Clinical and Hematological Features" @default.
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• W2093441092 doi "https://doi.org/10.1159/000320271" @default.
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